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治疗获得性血友病A患者急性出血发作的干预措施。

Interventions for treating acute bleeding episodes in people with acquired hemophilia A.

作者信息

Zeng Yan, Zhou Ruiqing, Duan Xin, Long Dan, Yang Songtao

机构信息

Department of Hematology, General Hospital of Chengdu Military Region, 270#, Rongdu Da Dao, Chengdu, Sichuan, China, 610083.

出版信息

Cochrane Database Syst Rev. 2014 Aug 28;2014(8):CD010761. doi: 10.1002/14651858.CD010761.pub2.

Abstract

BACKGROUND

Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies to coagulation factor VIII (FVIII). In most cases, bleeding episodes are spontaneous and severe at presentation. The optimal hemostatic therapy is controversial.

OBJECTIVES

To determine the efficacy of hemostatic therapies for acute bleeds in people with acquired hemophilia A; and to compare different forms of therapy for these bleeds.

SEARCH METHODS

We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library 2014, Issue 4) and MEDLINE (Ovid) (1948 to 30 April 2014). We searched the conference proceedings of the: American Society of Hematology; European Hematology Association; International Society on Thrombosis and Haemostasis (ISTH); and the European Association for Haemophilia and Allied Disorders (EAHAD) (from 2000 to 30 April 2014). In addition to this we searched clinical trials registers.

SELECTION CRITERIA

All randomised controlled trials and quasi-randomised trials of hemostatic therapies for people with acquired hemophilia A, with no restrictions on gender, age or ethnicity.

DATA COLLECTION AND ANALYSIS

No trials matching the selection criteria were eligible for inclusion.

MAIN RESULTS

No trials matching the selection criteria were eligible for inclusion.

AUTHORS' CONCLUSIONS: No randomised clinical trials of hemostatic therapies for acquired hemophilia A were found. Thus, we are not able to draw any conclusions or make any recommendations on the optimal hemostatic therapies for acquired hemophilia A based on the highest quality of evidence. GIven that carrying out randomized controlled trials in this field is a complex task, the authors suggest that, while planning randomised controlled trials in which patients can be enrolled, clinicians treating the disease continue to base their choices on alternative, lower quality sources of evidence, which hopefully, in the future, will also be appraised and incorporated in a Cochrane Review.

摘要

背景

获得性血友病A是一种由针对凝血因子VIII(FVIII)的自身抗体引起的罕见出血性疾病。在大多数情况下,出血发作在就诊时是自发且严重的。最佳止血治疗存在争议。

目的

确定获得性血友病A患者急性出血的止血治疗效果;并比较这些出血的不同治疗形式。

检索方法

我们检索了Cochrane对照试验中心注册库(CENTRAL)(《Cochrane图书馆》2014年第4期)和MEDLINE(Ovid)(1948年至2014年4月30日)。我们检索了以下会议的会议记录:美国血液学会;欧洲血液学协会;国际血栓与止血学会(ISTH);以及欧洲血友病及相关疾病协会(EAHAD)(2000年至2014年4月30日)。除此之外,我们还检索了临床试验注册库。

入选标准

所有针对获得性血友病A患者止血治疗的随机对照试验和半随机试验,对性别、年龄或种族无限制。

数据收集与分析

没有符合入选标准的试验可纳入。

主要结果

没有符合入选标准的试验可纳入。

作者结论

未找到针对获得性血友病A止血治疗的随机临床试验。因此,我们无法基于最高质量的证据就获得性血友病A的最佳止血治疗得出任何结论或提出任何建议。鉴于在该领域开展随机对照试验是一项复杂的任务,作者建议,在规划可纳入患者的随机对照试验时,治疗该疾病的临床医生继续基于其他质量较低的证据来源做出选择,希望未来这些证据也能得到评估并纳入Cochrane系统评价。

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本文引用的文献

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Acquired Factor VIII Inhibitors: Three Cases.获得性因子 VIII 抑制剂:三例报告。
Turk J Haematol. 2013 Mar;30(1):76-80. doi: 10.4274/tjh.2012.0009. Epub 2013 Mar 5.
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Acquired haemophilia A: a 2013 update.获得性血友病 A:2013 年更新。
Thromb Haemost. 2013 Dec;110(6):1114-20. doi: 10.1160/TH13-05-0363. Epub 2013 Sep 5.
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How I manage patients with acquired haemophilia A.如何管理获得性血友病 A 患者。
Br J Haematol. 2013 Apr;161(2):157-65. doi: 10.1111/bjh.12228. Epub 2013 Feb 4.

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